Τίτλος:
Practical recommendations for the diagnosis and management of transthyretin cardiac amyloidosis
Γλώσσες Τεκμηρίου:
Αγγλικά
Περίληψη:
Cardiac amyloidosis (CA) is an infiltrative restrictive cardiomyopathy caused by accumulation in the heart interstitium of amyloid fibrils formed by misfolded proteins. Most common CA types are light chain amyloidosis (AL) caused by monoclonal immunoglobulin light chains and transthyretin amyloidosis (ATTR) caused by either mutated or wild-type transthyretin aggregates. Previously considered a rare disease, CA is increasingly recognized among patients who may be misdiagnosed as undifferentiated heart failure with preserved ejection fraction (HFPEF), paradoxical low-flow/low-gradient aortic stenosis, or otherwise unexplained left ventricular hypertrophy. Progress in diagnosis has been due to the refinement of cardiac echocardiographic techniques (speckle tracking imaging) and magnetic resonance (T1 mapping) and mostly due to the advent of bone scintigraphy that has enabled noninvasive diagnosis of ATTR, limiting the need for endomyocardial biopsy. Importantly, proper management of CA starts from early recognition of suspected cases among high prevalence populations, followed by advanced diagnostic evaluation to confirm diagnosis and typing, preferentially in experienced amyloidosis centers. Differentiating ATTR from other types of amyloidosis, especially AL, is critical. Emerging targeted ATTR therapies offer the potential to improve outcomes of these patients previously treated only palliatively. © 2021, The Author(s), under exclusive licence to Springer Science+Business Media, LLC part of Springer Nature.
Συγγραφείς:
Bistola, V.
Parissis, J.
Foukarakis, E.
Valsamaki, P.N.
Anastasakis, A.
Koutsis, G.
Efthimiadis, G.
Kastritis, E.
Περιοδικό:
Heart Failure Reviews
Λέξεις-κλειδιά:
acoramidis; amino terminal pro brain natriuretic peptide; amyloid protein; biological marker; doxycycline; immunoglobulin light chain; monoclonal antibody; placebo; prx 004; taurursodiol; technetium 99m; tracer; transthyretin; unclassified drug; transthyretin, AL amyloidosis; aortic stenosis; ATTR amyloidosis; biopsy technique; bone scintiscanning; cardiovascular magnetic resonance; clinical feature; disease classification; disease surveillance; drug efficacy; echocardiography; electrocardiography; gene mutation; genetic analysis; heart amyloidosis; heart failure with preserved ejection fraction; heart interstitium; heart left ventricle hypertrophy; heart muscle biopsy; heart transplantation; human; intracardiac echocardiography; liver transplantation; myocardial perfusion imaging; positron emission tomography; prevalence; rare disease; restrictive cardiomyopathy; Review; cardiomyopathy; familial amyloid polyneuropathy; heart; heart failure; heart stroke volume, Amyloid Neuropathies, Familial; Cardiomyopathies; Heart; Heart Failure; Humans; Prealbumin; Stroke Volume
DOI:
10.1007/s10741-020-10062-w
